Inflamm Bowel Dis. 2020 May 9;izaa080. doi: 10.1093/ibd/izaa080. Online ahead of print.

Basavaraj Kerur ¹, Eric I Benchimol ², Karoline Fiedler ³, Marisa Stahl ⁴, Jeffrey Hyams ⁵, Michael Stephens ⁶, Ying Lu ⁷, Marian Pfefferkorn ⁸, Raza Alkhouri ⁹, Jennifer Strople ¹⁰, Judith Kelsen ¹¹, Leah Siebold ¹², Alka Goyal ¹³, Joel R Rosh ¹⁴, Neal LeLeiko ¹⁵, Johan Van Limbergen ¹⁶, Anthony L Guerrerio ¹⁷, Ross Maltz ¹⁸, Lina Karam ¹⁹, Eileen Crowley ²⁰, Anne Griffiths ³, Melvin B Heyman ²¹, Mark Deneau ²², Keith Benkov ²³, Joshua Noe ²⁴, Dedrick Mouton ²⁵, Helen Pappa ²⁶, Joseph A Galanko ²⁷, Scott Snapper ²⁸, Aleixo M Muise ³, Michael D Kappelman ²⁷

Author information

¹University of Massachusetts Medical School (UMMS), Worcester, MA, United States.

²Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.

³The Hospital for Sick Children, Toronto, ON, Canada.

⁴Children's Hospital Colorado, Denver, CO, United States.

⁵Connecticut Children's Medical Center, Hartford, CT, United States.

⁶Center for Individualized Medicine, Mayo Clinic, Rochester, MN, United States.

⁷Cohen Children's Medical Center of New York, New York, NY, United States.

⁸Riley Children's Hospital, Indianapolis, IN, United States.

⁹University at Buffalo, Buffalo, NY, United States.

¹⁰Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, United States.

¹¹Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States.

¹²UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, United States.

¹³Children's Mercy, Kansas City, MO, United States.

¹⁴Goryeb Children's Hospital/Atlantic Health System, Morristown, NJ, United States.

¹⁵Hasbro Children's Hospital, Providence, RI, United States.

¹⁶Amsterdam University Medical Centres, Emma Children's Hospital, Amsterdam, Netherlands.

¹⁷Johns Hopkins University School of Medicine Baltimore, MD, United States.

¹⁸Nationwide Children's Medical Center, Columbus, OH, United States.

¹⁹Texas Children's Hospital, Houston, TX, United States.

²⁰Children's Hospital, London Health Science Centre, University of Western Ontario, London, Ontario, Canada.

²¹UCSF Benioff Children's Hospital, San Francisco, CA, United States.

²²University of Utah/Intermountain Primary Children's Hospital, Salt Lake City, UT, United States.

²³Icahn School of Medicine at Mount Sinai, New York, NY, United States.

²⁴Medical College of Wisconsin, Milwaukee, WI, United States.

²⁵Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN, United States.

²⁶Cardinal Glennon Children's Hospital, St. Louis, MO, United States.

²⁷University of North Carolina in Chapel Hill, Chapel Hill, NC, United States.

²⁸Children's Hospital Boston, Boston, MA, United States.

Abstract

Background: The incidence of very early onset inflammatory bowel disease (VEOIBD) is increasing, yet the phenotype and natural history of VEOIBD are not well described.

Methods: We performed a retrospective cohort study of patients diagnosed with VEOIBD (6 years of age and younger) between 2008 and 2013 at 25 North American centers. Eligible patients at each center were randomly selected for chart review. We abstracted data at diagnosis and at 1, 3, and 5 years after diagnosis. We compared the clinical features and outcomes with VEOIBD diagnosed younger than 3 years of age with children diagnosed with VEOIBD at age 3 to 6 years.

Results: The study population included 269 children (105 [39%] Crohn's disease, 106 [39%] ulcerative colitis, and 58 [22%] IBD unclassified). The median age of diagnosis was 4.2 years (interquartile range 2.9-5.2). Most (94%) Crohn's disease patients had inflammatory disease behavior (B1). Isolated colitis (L2) was the most common disease location (70% of children diagnosed younger than 3 years vs 43% of children diagnosed 3 years and older; P = 0.10). By the end of follow-up, stricturing/penetrating occurred in 7 (6.6%) children. The risk of any bowel surgery in Crohn's disease was 3% by 1 year, 12% by 3 years, and 15% by 5 years and did not differ by age at diagnosis. Most ulcerative colitis patients had pancolitis (57% of children diagnosed younger than 3 years vs 45% of children diagnosed 3 years and older; P = 0.18). The risk of colectomy in ulcerative colitis/IBD unclassified was 0% by 1 year, 3% by 3 years, and 14% by 5 years and did not differ by age of diagnosis.

Conclusions: Very early onset inflammatory bowel disease has a distinct phenotype with predominantly colonic involvement and infrequent stricturing/penetrating disease. The cumulative risk of bowel surgery in children with VEOIBD was approximately 14%-15% by 5 years. These data can be used to provide anticipatory guidance in this emerging patient population.